Living and Dying With Muscular Dystrophy

It felt like I was interviewing my brother. The shape of his head and torso were identical. The chubby cheeks and sneaky smile were the same. And the endless attempts to shift his bulbous arms and get comfortable in his wheelchair were an exact replica of Tommy.

But this wasn't my brother. Tommy sadly passed away in January 1991. This was Darius Weems, from Athens, Ga., and this is what Duchenne muscular dystrophy, or DMD, does to the body.

I met Darius at the Santa Barbara Film Festival, when I attended the world premiere of his remarkable documentary, "Darius Goes West."

The film tells the story of his first-ever trip beyond the boundaries of his hometown. He and a team of friends/helpers rent an RV and drive from Georgia to California. Their intention is to visit West Coast Customs, the company featured in MTV's "Pimp My Ride," in the hope that Darius' wheelchair would be tricked out just like the cars on his favorite show. In the process, they raise awareness about a condition that remains the single biggest genetic killer of children on the planet.

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The documentary records a journey that is full of happiness and heartbreak.

Darius, who'd never previously seen the ocean, is carried out of his wheelchair on one occasion and helped to stand in the sea. The miracle of being upright again is not lost on him or his friends, and Darius bursts into uncontrollable laughter as the waters crash against his skin.

And then there are times when he can't get into a roadside gas station because there is no access for his wheelchair. He repeatedly crashes his wheelchair into the curbside, but to no avail. His frustration is loud and deep.

It was impossible to watch the film without seeing my brother in every frame.

He too was born with DMD in 1962 -- 13 months before my birthday. It's a genetic disease that, according to the National Institutes of Health Web site, affects around one in every 3,500 male births worldwide -- almost solely boys -- and is characterized by rapidly worsening muscle weakness in the legs and pelvis, and eventually affects the whole body. When Tommy was Darius' current age, 17, he too appeared to possess the body of an over-eager bodybuilder. All his muscles were bulging -- and completely useless.

It is a cruel condition in that sufferers begin their lives like any other child -- walking, talking and in the case of Darius, shooting hoops. But then, from the age of 5 or 6, they begin to slow down.

When Tommy was about 7, I began to realize that stealing his toys or beating him in races was getting easier. At the time, I had no idea that his spirit was willing but his flesh was weakening. I still bitterly regret those moments of sibling rivalry because he stood no chance of winning against his younger brother.

By the age of 10, Tommy and Darius both required walkers. Within two years they would become reliant upon their wheelchairs. Their similarities are just as consistent for almost every child with the disease. And the final reality is that the majority die by the age of 25.

But now, for the first time since the disease was described by French neurologist Guillaume Duchenne during the 1860s, scientists are inching closer to a major breakthrough -- even a cure.

A Dutch biotech firm, Prosensa, is conducting human clinical trials of a therapy called exon skipping. This approach would transform DMD into the much milder form of Becker muscular dystrophy, which may allow sufferers to continue walking into their 40s.

Another biotech company, in North Carolina, Asklepios, is conducting a replacement therapy trial that aims to get a healthy copy of the defective gene into all the body's muscle cells, thus preventing the muscular and mortal effects of the disease. These research scientists say they are on the cusp of a cure.

Toward the end of "Darius Goes West," another young man is introduced. His name is Charley Seckler. He's about 6 years old and also has DMD. His father, Benjy, is a doctor. His mother, Tracey, is a teacher. Charley is still kicking a soccer ball, albeit slowly. They've started Charley's Fund to financially assist scientists around the world in their pursuit of a cure for DMD.

They told me that their own son might yet benefit from a breakthrough. After attending the premiere, they wondered whether he, too, would be able to stand in the ocean as a teenager, without being held up by his parents.

Of course, all these magnificent scientific breakthroughs will come too late for Darius -- and Tommy. But in their own ways, they have defied the odds, confronted insurmountable challenges and displayed courage beyond compare.

As I left the premiere, I suddenly remembered that I had watched a documentary on my last visit to Santa Barbara, back in 2005. I had been subpoenaed to appear in court during the Michael Jackson child abuse proceedings of 2005. As I sat in the dock, my documentary "Living with Michael Jackson" was played before the jury. I remain proud of that documentary, and I recall seeing even Judge Melville subtly nodding his head to some of Jackson's magnificent music.